Brain Abnormalities and Epilepsy in Patients with Parry-Romberg Syndrome

Area of Science:

• Neurology
• Radiology
• Genetics

Background:

• Parry-Romberg syndrome is a rare disorder causing progressive hemifacial atrophy.
• Brain abnormalities and epilepsy are known associations, but their spectrum is unclear.

Purpose of the Study:

• To characterize brain abnormalities in Parry-Romberg syndrome.
• To investigate the association between brain abnormalities and epilepsy in PRS patients.

Main Methods:

• Retrospective review of 80 patients with Parry-Romberg syndrome and brain MR imaging.
• Grading of unilateral hemispheric atrophy, white matter disease, microhemorrhage, and leptomeningeal enhancement.
• Analysis of MR imaging findings in relation to epilepsy and PRS side.

Main Results:

• 60% of patients had brain abnormalities; 32% were ipsilateral to facial atrophy.
• Epilepsy was present in 20% of patients.
• Brain abnormalities were more frequent (100% vs 48%) and ipsilateral (81% vs 20%) in epilepsy patients. White matter disease and hemispheric atrophy were predominant, more severe in epilepsy.

Conclusions:

• Ipsilateral brain abnormalities are common in Parry-Romberg syndrome.
• Epilepsy is associated with more frequent and severe brain abnormalities, particularly white matter disease and hemispheric atrophy.